0:14 - Definition of pulmonary hypertension 0:20 - Mean pulmonary artery pressure 1:00 - Using systolic to estimate pulmonary hypertension with echocardiogram 1:18 - 5 different WHO pulmonary hypertension groups 1:30 - Pulmonary arteriolar hypertension (PAH), Idiopathic pulmonary hypertension, collagen vascular diseases, portal HTN 3:35 - BMPR2 4:00 - Left heart failure 4:10 - Lung disease, COPD, OSA, idiopathic pulmonary fibrosis 4:35 - Pulmonary embolism, chronic VTE 4:50 - Hematologic disorders, sarcoidosis, glycogen storage diseases, renal failure 5:40 - Amphetamines 5:50 - Diagnosis of pulmonary hypertension and physical exam findings 6:07 - Heart sounds, loud P2, tricuspid regurgitation, RV heave 6:35 - JVP, c,v waves liver pulsatile, edematous legs 6:58 - Chest Xray findings with pulmonary hypertension 7:36 - ECG/EKG findings with pulmonary hypertension, RVH, RBBB 8:17 - Echocardiogram findings with pulmonary hypertension 9:03 - Tricuspid regurgitation, regurgitant jet, Modified Bernoulli Equation 10:40 - Advantages of echocardiogram: PASP, 11:10 - Echo bubble study 11:25 - Right heart catheter, wedge pressure, Left atrial pressure (LAP), mean artery pressure (MAP)
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Content
2.28 -> well welcome to another MedCram
lecture we're going to talk about
4.62 -> pulmonary hypertension treatment and
there's actually several different types
9.84 -> of treatments remember there are
different waho groups there's Group 1 2
16.17 -> 3 4 & 5
now group 1 we said was idiopathic
23.25 -> pulmonary arterial or hypertension and
the other ones for instance the collagen
30.21 -> vascular diseases the drugs the toxins
those sorts of things number one is
35.489 -> where most of the work in terms of drugs
specifically for pulmonary hypertension
41.399 -> have centered there's also one that is
FDA approved for four which we'll talk
45.66 -> about
but remember why this is is because
47.85 -> group two is almost exclusively is
exclusively due to left ventricular
53.16 -> failure Group three has to do with lung
disease and group five is kind of a
58.44 -> grab-bag so these already have their
treatments specifically for them one has
64.049 -> a whole bunch which we're going to talk
about and there is one that is indicated
67.799 -> for for remember for is for venous
thromboembolism or chronic pulmonary
74.57 -> hypertension or as it's officially
called chronic thromboembolic pulmonary
87.38 -> hypertension and that's abbreviated c t
e p h okay so let's talk about number
97.259 -> one and the different types of
treatments
100.159 -> okay so there's some things that all
groups would do well to start and we're
105.36 -> going to make this as simple as possible
and explain it as clearly as possible as
109.59 -> we normally do so because of the fact
that they get this lower extremity
113.27 -> diuretics is something that you want to
do the other thing that you want to do
119.759 -> if they need it is oxygen therapy and
they've noticed on autopsies that there
126.659 -> are a lot of blood clots not just in
group chronic thromboembolic pulmonary
133.17 -> but also in the other groups after many
studies anticoagulation was found to be
143.28 -> definitely something that you want to do
in group 4 and maybe in group number one
151.97 -> other medications that might be
beneficial is the Jaques in' and
157.55 -> exercise definitely beneficial so these
are ones that can go really to just
164.37 -> about all classes let's talk about group
one now and the different classes now
172.68 -> before we start with the advanced
medications that some of you are
176.1 -> familiar with we have to do something
called a vaso reactivity test and the
184.11 -> reason why we do this is because those
who respond to the vaso reactivity test
188.67 -> are more likely to respond to ordinary
medications like calcium channel
193.62 -> blockers like the dihydropyridine and
diltiazem the calcium channel blockers
198.989 -> so if they respond if that's a positive
response calcium channel blockers if
203.73 -> it's negative then we go on down to the
more advanced medications so what is the
211.019 -> base of reactivity test looking at well
there's a number of ways you can do it
214.5 -> you can use nitric oxide to see whether
or not the patient's mean pulmonary
219.48 -> artery pressure drops by 10 millimeters
of mercury the other thing that you can
224.67 -> do is you can use equal process in all
229.639 -> finally the other thing that you can use
is adenosine so all of these are
235.01 -> medications that can be used to see
whether or not the patient is reactive
241.26 -> and once again it's considered positive
if the main pulmonary artery pressure
245.4 -> decreases by at least 10 millimeters of
mercury and goes to less than 40
250.23 -> millimeters of mercury this is assuming
that the cardiac output actually gets
255.48 -> better or it's unchanged and as we
mentioned patients with a positive
260.849 -> reactivity tests are ones that could
improve with calcium channel blockers
265.83 -> those that are Nega
will not respond to calcium channel
269.61 -> blockers so once we do this and I'll
tell you it's a very small percent are
274.14 -> actually reactive most of these are
going to go into the negative category
277.2 -> and so most of them are going to be
having to be put on advanced medications
280.92 -> if we're dealing with a group one so
let's talk about those things but before
285.36 -> we do the thing that's going to
determine what medication they get is to
289.62 -> determine how sick they really are and
that's determined by a w-h-o again
294.56 -> functional class and so there's
functional class 1 which is the most
301.68 -> mild and they typically don't need
medications that can be monitored and
306.36 -> then there's class
- and there's class 3 and these are
311.7 -> where most of the medications are
actually started and then there's class
316.32 -> 4 which is the most severe and these are
usually given IV so the way I remember
322.32 -> it is that class IV should be given IV
class 1 doesn't need to be giving any
327.72 -> medications orally and the ones in the
middle are given Pio meds and those Pio
333.39 -> meds we'll talk about very shortly most
of these medications haven't been around
337.98 -> for more than 15 years that's how new
they are and if you're lucky enough to
342.84 -> have a patient who is responsive to a
Veysel reactive medication then you can
348.06 -> just give a calcium channel blocker
which is dirt cheap and has been around
350.52 -> for many years if on the other hand they
don't respond and there are w-h-o class
355.89 -> 2 or 3 then you're going to be starting
them on some Pio meds which we're gonna
360.03 -> talk about right now the first category
is the prostacyclin agonists so this is
366.36 -> like equal process and all IV which is
indicated for stage 4 IV as we talked
372.87 -> about there's tree procced Annelle which
can come IV sub-q or inhaled there's
377.97 -> Isla Pross which is comes inhaled and
then there's these prostacyclin agonists
382.77 -> which are not really prostacyclin x'
like celexa peg which all of these
388.11 -> things stimulate the prostacyclin
receptor and in effect caused an
394.05 -> increase in cyclic AM P and therefore
vaso dilation
401.159 -> okay so those are the prostacyclin
agonists the next group are the endo
408.219 -> feelin receptor antagonists so endo
feelin one is a hormone that basically
414.369 -> circulates around and is extremely
potent vasoconstrictor so if we could
418.839 -> possibly block these receptors
potentially we could get some basal
423.789 -> dilation that's exactly what we see
there's two types of receptors is the a
428.169 -> and B and so both senton and massive
Tenten is basically a drug that blocks
434.169 -> these receptors it's non-selective and
it seems to reduce the PA pressures it
439.089 -> also like a lot of the other medications
improves the quality of life extends the
444.699 -> length of time before decompensation and
increases exercise capacity one of the
450.849 -> selective ones is amber senton so these
are the receptor field and receptor
455.86 -> antagonists these are a list of
medications that are fda-approved for
461.619 -> pulmonary hypertension specifically
sildenafil and Tel dalla fill the
466.569 -> purpose of these things is to inhibit
the breakdown of these medications which
471.909 -> basically increase the amount of nitric
oxide so there is cyclic GMP and that
482.349 -> cyclic quantity monophosphate is broken
down by phosphodiesterase well these
487.569 -> medications inhibit the ability of this
phosphodiesterase to break down the cmp
493.329 -> and so these are why they're called pde5
inhibitors and so what happens is cyclic
499.659 -> GMP goes up which stimulates an increase
in nitric oxide and it's nitric oxide
504.969 -> which is a vasodilator okay so there's a
couple of other things that also
511.469 -> increase vasodilation and that would be
like an alpha blocker or a nitrate so
517.959 -> you should not be on these medications
at the same time these pb5 inhibitors
522.219 -> cause vasodilation in the pulmonary
vasculature by increasing nitric oxide
527.649 -> and they do it specifically by
inhibiting the breakdown of cyclic GMP
534.46 -> the last mechanism that we're going to
look at is the guanylate cyclase direct
539.29 -> stimulants and this riociguat is one of
the medications that is fda-approved is
545.77 -> the medication that is FDA approved to
do this and it's a direct stimulator of
551.279 -> the nitric oxide receptor so it
increases nitric oxide just like the
558.18 -> phosphodiesterase inhibitors do but they
do it in a different way and they have a
563.41 -> dual mode of action not only do they
increase the nitric oxide receptor they
569.35 -> also increase the sensitivity of the SGC
to endogenous nitric oxide which is a
575.83 -> pulmonary vasodilator and so they also
directly stimulate the receptor to mimic
579.55 -> the action of nitric oxide so this is a
little different and not only is it
584.11 -> approved in a pH group w-h-o group
number one but it's also approved for
595.62 -> w-h-o group number four which remember
is the chronic thromboembolic disease so
601.6 -> just be aware of that now all of the
medications that we've just talked about
605.529 -> can be used in combination but you've
got to be careful make sure again that
612.64 -> you are using these in combination after
you have done a right heart cath and
618.16 -> made sure that they are not reactive so
you could add for instance tadalafil and
624.459 -> amber senton you could use sildenafil
and both senton you could use both
630.279 -> senton added to either Ypres process and
all or triple process Annelle or you
635.47 -> could do triple process Annelle added to
either both sent in or sildenafil so
639.279 -> there's different ways of doing this and
this is very similar to how we increase
643.39 -> blood pressure medication for systemic
hypertension we can also do this for
647.62 -> pulmonary hypertension so the key points
again are that if you suspect somebody
653.02 -> of having pulmonary hypertension make
sure that you classify them in a w-h-o
657.79 -> group if they are group 1 or 4 there may
be medication specifically designed for
663.49 -> these types of diseases if it is group 2
you need to
667.84 -> get the underlying cause of the left
heart disease and treat that if it is
671.35 -> three you have to look at the underlying
cause for the hypoxemia and treat that
675.88 -> whether it's lung disease or sleep apnea
for you need to anticoagulate them
681.16 -> generally and they may need to go to
surgery where they actually have the
684.73 -> clot removed or if that's not feasible
they may have to consider a medication
689.29 -> like we just talked about here and then
five is kind of the grab bag it should
693.34 -> be looked at in terms of the other
causes so that is a very brief primer in
699.28 -> pulmonary hypertension how it is
diagnosed how it is looked at how it is
705.01 -> treated and and what are the medications
that are involved so thanks for joining
